Cuvrior (trientine tetrahydrochloride)

Cuvrior (trientine tetrahydrochloride)

Self-Administration – Oral

Diagnosis considered for coverage:

• Wilson's Disease: Indicated for the treatment of adult patients with stable Wilson's disease who are de-coppered and tolerant to penicillamine.

Coverage Criteria:

For diagnosis of Wilson's disease:

• Dose does not exceed 3,000 mg per day, AND

• Diagnosis of Wilson's disease (i.e., hepatolenticular degeneration), AND

• Prescribed by or in consultation with one of the following:

  • Gastroenterologist

  • Hepatologist, AND

• Documentation of one of the following:

  • Presence of Kayser-Fleisher rings

  • Serum ceruloplasmin (CPN) less than 20 mg/dL

  • 24-hour urinary copper excretion greater than 100 mcg

  • Liver biopsy with copper dry weight greater than 250 mcg/g

  • ATP7B mutation via genetic testing, AND

• Trial and failure, or intolerance to Depen (penicillamine) tablets

Reauthorization Criteria:

For diagnosis of Wilson's disease:

• Documentation of a positive clinical response to therapy

Coverage Duration:

• Initial: 12 months

• Reauthorization: 12 months

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Policy Updates:

11/14/2023 – New policy approved by P&T.

References:

1. Pearle MS, Goldfarb DS, Assimos DG, et al. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug;192(2):316-24.

2. Fattah H, Hambaroush Y, Goldfarb DS. Cystine nephrolithiasis. Transl Androl Urol. 2014 Sep 1;3(3):228-233. doi: 10.3978/j.issn.2223-4683.2014.07.04.

3. European Association for Study of Liver. EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012;56(3):671-685.

4. Cuvrior Prescribing Information. Orphalan SA. Chicago, IL. May 2022.